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NEUROLOGY 1985;35:1355
© 1985 American Academy of Neurology

ATP degradation products after ischemic exercise

Hereditary lack of phosphorylase or carnitine palmityltransferase

Tulio E. Bertorini, Vera Shively, Bonnie Taylor, Genaro M.A. Palmieri and Irving H. Fox

From the Human Purine Research Center, Departments of Internal Medicine and Biological Chemistry, Clinical Research Center, University Hospital, Ann Arbor, MI, and the Departments of Neumlogy and Medicine, University of Tennessee Center for the Health Sciences, Memphis, TN.

We measured purine degradation products of ATP in plasma after ischemic exercise in eight normal subjects, one patient with myo-phosphorylase deficiency (McArdle's disease), and one with carnitine palmityltransferase deficiency. Normal subjects increase hypoxanthine and inosine, but not xanthine. Plasma purine levels were elevated above the normal range after ischemic exercise in McArdle's disease and in carnitine palmityltransferase deficiency after fasting. Those changes implied abnormally accelerated ATP degradation in these two myopathies.

Address correspondence and reprint requests to Dr. Fox, Clinical Research Center, University Hospital, Ann Arbor, MI 48109.

Supported by a grant from the Muscular Dystrophy Association, Inc., and from the USPHS 5-MO1 RR42. R01 AM 19674, and 5–M01 RR22.

Accepted for publication December 11, 1984.







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